ANAL RECTAL MALFORMATIONS

(ARMs)

PRESENTER:- SR. MASSENGA

GENERAL SURGERY DEPARTMENT

 SUBTOPICS
 LIST OF ABBREVIATION

 DEFINITION

 EPIDEMIOLOGY

 CLASSIFICATION

 CLINICAL PRESENTATION

 ASSOCIATED ANOMALIES

 INVESTIGATION

 TREATMENT

 COMPLICATIONS

 BMC CHALLENGES
 LIST OF ABBREVIATIONS
• ARM…Anal rectal malformation
• PSARP… posterior sagittal anorectoplasty
• CMF…Congenital malformation
• ASD…Atrial septal defect
• PDA…Patent ductus arteriosus
• FOF…Tetralogy of Fallot
• VSD…Ventricular septal defect
• NGT…Nasal gastric tube
• Abd uss…Abdominal ultrasound
• ECHO…
• NMRI…Nuclear magnetic resonance imaging
 Definition
• ARMs
are birth defects in which the rectum and anus are
malformed, do not develop properly.

ARMs
happen as a fetus is developing during pregnancy.

• During a bowel movement, stool passes from the

large intestine to the rectum and then to the anus.
 ROUTES

Colon Rectum Anus

Males Female

RVF
RVF

RUF

RSF

RVF Rectal Terminus

 INCIDENCE

• Most authors have written that the average

incidence worldwide is 1 in 5,000 live births.

• Some authors have shown relationship btn families

and ARM. There is genetic predisposition with
ARMs being diagnosed in succeeding generations.
Not yet confirmed.

The causes of anorectal malformation are unknown.

 QUESTION

DO WE HAVE
PATIENTS WITH
ARMs?
 Total No. of Total No. of pts Total No. of Total no of
YEAR 2014 surgical with CMF patients with operated patients
pediatric pts ARM with ARM

JAN 252 85 13 3
FEB 297 97 16 5
MARCH 212 86 11 8
APRIL 244 116 10 6
MAY 290 104 9 8
JUNE 357 122 20 6
JULY 416 92 11 8
AUGUST 397 120 17 4

SEPTEMBER 314 133 18 5

OCTOBER 385 140 14 6
NOVEMBER 379 96 25 5
DECEMBER 334 92 11 9
TOTAL 3872 1283 175 75
 BMC DATA 2014

1. 6% ARMs out of surgical pediatric pts

2. 13.6% ARM out of other CMF

3. 40.6% operated out of ARMs

• Why 49.4% were not operated?

 BMC DATA 2015

40 Children

9 Children

5 Children 1° surgeries

4 Children= 10% definitive surgeries

WHY THE REST WERE NOT OPERATED?

 BMC CHALLENGES 2015
1.Diagnostic tools
i. Fluoroscopy machine was not working
Cloaca 4patients(5, 3, 2,1) vascular contrast
Other types of ARM, barium enema

ii. Fresh frozen section machine

Some children had Hirschsprung‘s disease

iii. Rectal bleeding. Endoscopy; Low GI

examinations/ Pediatric scopy
2. AGE; >1yr
 PATHOGENESIS

• Embryology

• Hindgut.

• When various steps in the process of development

of organs (organogenesis) fail, anal malformations
occur.

• The exact etiology of this failure has not yet

identified.
 1.CLASSIFICATION OF ARMs
• Described at the WINGSPREAD CONFERENCE U.S.A in
1994
1. High,
2. Intermediate
3. Low ARM in relationship to the level of the
puboretalis portion of the levator ani Muscles.

For the purpose of simplifying the treatment, lesions

were classified only as
High or
Low ARM depending on the position of the
puboretalis portion of the levator ani Muscles
 ARM
Pelvic
diaphragm
is composed
of 3 muscles
HIGH OR LOW ARM
HIGH OR ARMs
2. CLASSIFICATION OF ARM, INVERTOGRAM
 3. CLASSIFICATION OF ARM
FEMALES MALES
Rectoperineal fistula Rectoperineal fistula

Rectovestibular fistula Rectourethral fistula

Bulbar
Prostatic

Persistent cloaca Rectovesical fistular

<3cm common channel
>3cm common channel
Imperforate anus withought Imperforate anus withought
fistula fistula
Rectal atresia Rectal atresia
 ARMDIAGRAMATICALLY
• Anal atresia
• Membranous anus
• Rectoscrotal
• Rectovestibular anus
• Rectal vesical
• Rectourethral fistula
• Anterior positioned anus
• Posterior positioned anus
• Rectovaginal
• Rectoperineal
• Bucket handle deformity
• Anal sternosis
• Rectal atresia
ARM
ARM
ARM
ARM
 ARM

RECTAL ATRESIA
ARM

RVF
ARM Bucket handle deformity
ARMCLOACA
 ARM
Cloaca.
a situation that has urethral,
vaginal and rectal openings all
sharing a common single external
orifice.

Single perineal opening

Cloacas with common

channel/chamber, holds fecal
matter, urine and reproductive fluid,
they are eliminated through a single
opening

Common channel less than 3cm

Good outcome >3cm

ARMBEZARRE
 ARM

• The most common defect in females is imperforated

anus with rectovestibular fistula

• whereas the common defect in males is

imperforated anus with rectourethral fistula
 CLINICAL PRESENTATION
Thorough perineal inspection

• 1. If meconium is seen on perineum, it is evidence

of a rectoperineal fistula.

• 2. If meconium is in the urine, the diagnosis of a

rectourinary fistula is obvious.
1+2= low lesion
 CLINICAL PRESENTATION

• Imperforate anus is a clinical diagnosis based on

careful inspection of the perineum.
No anal opening…imperforate anus

• Single perineal opening…Cloaca

Low imperforate anus in a male. “Bucket
handle” shown at site
of covered anus with small amount of
meconium extruding (arrow).
LOW ARM
Well-developed median raphe and
anal dimple.
 HIGH ARM

• Perineum is not stained with meconeum

• High imperforate anus in a male. Median raphe is

flat (not well formed)and without any signs of
meconium extrusion.

• No anal dimple
HIGH ARM
 ASSOCIATED MALFORMATIONS
Approximately 60% of patients have an associated
malformation.
VACTERL
• V…Vertebral and spinal cord malformations
• A…ARM
• C…Cardiac
• TE…TracheoEsophageal anomalies
• R…Renal and other urinary tract malformations
• L…Limb malformations
 ASSOCIATED MALFORMATIONS
• Many associated malformations are incidental
findings, but other malformations like cardiac
malformations may be life threatening

"If any one of these anomalies is discovered, it is

essential to search for the others."
 ASSOCIATED MALFORMATIONS

• Cardiac malformations CM

• 1/3 of the pts with ARM, have cardiac malformations.

The most common lesions are ASD, PDA followed by
the TOF and VSD defect.

• The two most common cardiac anomalies are TOF and

VSD defects.

• Other malformations like transposition of the great

arteries is rare.
 ASSOCIATED MALFORMATIONS
TracheoEsophageal anomalies
• Esophageal atresia
• TracheoEsophageal fistula,

Gastrointestinal anomalies
• Infantile hypertrophied pyloric sternosis
• Duodenal atresia,
• Duodenal web
• Malrotations
• Hirschsprung's disease

Abdominal wall anomalies

• Omphalocele
• Gastroschisis
 ASSOCIATED MALFORMATIONS
Vertebral and spinal anomalies

• Lumbosacral anomalies like butterfly

vertebrae, hermivetebrae, hermisacrum and
scoliosis are common.

• The most frequent spinal anomalies is

tethered cord, but spinal lipomas and
myelomeningocele are also common.
• "The most complex the anorectal anomaly,
the more likely the presence of an associated
vertebral and spinal anomaly"
 ASSOCIATED MALFORMATIONS
GENITOURINARY ANOMALIES

• The most common is vesicoureteric reflux the retrograde

flow of urine from the bladder into the ureter, or kidneys G1-v, followed by
renal agenesis, renal dysplasia internal structures of one or both
of the baby's kidneys do not develop normally.

• Others are cryptorchidism absence of one or both testes from the

scrotum. It is the most common birth defect of the male genitalia and
hypospadias urethral opening is ectopically located on the ventrum of the
penis proximal to the tip of the glans penis
 ASSOCIATED MALFORMATIONS
• LIMBS
Clubfeet
 INVESTIGATONS
• Invertogram

for imperforated anus

withought fistula.

a radiopaque marker on
the perineum at the anal
dimple.

Air acts as the contrast

agent, it shows the
location of the rectal
terminus.
 INVESTIGATONS
Colostogram
For imperforated
anus with fistula.
It shows
colourethral
fistula.

This is high
imperforate anus
in male
 INVESTIGATONS

• Retrograde barium enema/ vascular contrast

Lateral view
 OTHER INVESTIGATIONs
• NGT…Esophageal atresia,

• ECHO…Cardiac malformation

• Abdominal uss… Renal agenesis

• Plain X-ray films… vertebral anomalies

• Spine uss and lumbar NMRI for tethered cord

 TREATMENT

• Prophylactic antibiotics
• Colostomy-emergency vs elective

Definitive surgery-Elective

• Low ARM…PSARP
• High ARM…Pull down
• HD…Pullthrough
BMC EXPERIENCE
 TAKE HOME MESSAGE

ARMs are unpreventable.

They are surgically treatable.

 REFERENCES
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Numanoglu A, Banieghbal B, Chitnis M, Birabwa-Male D,
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2. Uba AF, Chirdan LB, Ardill W, Edino ST. Anorectal anomaly: a

review of 82 cases seen at JUTH, Nigeria. Niger Postgrad Med J
2006;13(1):61–65

3. Adejuyigbe O, Abubakar AM, Sowande OA, Olayinka OS, Uba

AF. Experience with anorectal malformations in Ile-Ife, Nigeria.
Pediatr Surg Int 2004; 20:855–858.

4. Archibong AE, Idika IM. Results of treatment in children with

anorectal malformations in Calabar, Nigeria. S Afr J Surg 2004;
42(3):88–90.

5. Johnson O, Ghidey Y, Habte D. Congenital malformation of anus

and rectum in Ethiopian children. Ethiop Med J 1981; 19(1):9–15.
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Publishers, 2003, Pp 739–761.

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Malformations. Springer-Verlag, 1989.

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cloacas. J Pediatr Surg 1997; 32:263–268.

9. Peña A, Guardino K, Tovilla JM. Bowel management for fecal

incontinence in patients with anorectal malformations. J Pediatr
Surg 1998; 33:133–137.

10. Malone PS, Ransley PG, Kiely EM. Preliminary report: the
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 TREATMENT
• A posterior sagittal anorectoplasty can then be
performed between 2 and 12months of age,
followed by colostomy reversal.

• At two weeks postoperatively the anus is sized with

a Hagar dilator (digital anal dilatation) and the
caregiver is instructed on frequent home
dilatations.

• The colostomy may then be closed in six weeks or

any convenient time after that.